"I AM"- are the two most powerful words in the dictionary because the ending determines your destiny….so join me in my fight against PD to make sure that everyone who suffers from this chronic progressive degenerative disease can develop the courage to shout to the wind- I AM Fierce and Courageous ….
Have you ever seen a water spout? A sudden breeze of wind elevates the water and makes it spin faster and faster in a matter of seconds until it gains enough momentum to glide on the surface and travel right out of its boundaries. I have stood at the edge of shore watching in awe how a once calm ocean suddenly spewed out a spinning water spout the size of a skyscraper. As it passed by my side scaring the heebie-jeebies out of me, it crossed the street, as if it owned it all along.
This is what having biphasic dose dyskenesias can be like. This is not a frequently occurring type of involuntary movements caused by levodopa intake, when it occurs it can be extremely unsettling to say the least. this is by far in my experience the most difficult to treat with medication. This is because patients experience dyskenisias of brief duration shortly after taking medication (within half hour to an hour) followed by severe spasms and dystonia particularly in lower extremities 1-2 hours later. This phenomena can occur through out the day as were mine in the evening. but, they are more commonly present in the am with first dose. Although once in a while we get lucky as was my case. For me decreasing the dose of medication in the evening did the trick. However, the best solution and treatment for people having this problem is deep brain stimulation.
Since all dyskenisias are not equal, you as patient and care giver can best advocate for yourself or loved one by keeping a close diary of events so that your physician /MDS can best adjust or decide plan of action to best suit your needs.
Recommendations: keep a diary of events:
total episodes you have in a typical day/ week/month
how many hours/ minutes before the dyskenesias take place after medicine intake
how many hours/ minutes do these episodes last?
after the episode how long before you started to feel well again/ have symptom control?
how long did you have symptom control with medicines?
did the symptom control last from dose to dose?
did you experience dyskenesias then spasms? How often and when?
also think about how many hours in the day you are well vs unwell?
Don’t forget to list any and all medications you take to cope with these symptoms when they occur. (any over the counter meds? home remedies? pain pills?)
How is your life impacted by these episodes
How often a week/month do you miss work/paid occupation or domestic responsibilities because you are experiencing dyskenesias?
How often do you miss social events weekly/monthly with family and friends because you don’t feel well due to the dyskenesias?
Are you unable to do or carry out your hobbies or passion? how often does this happen?
The day i could no longer go shopping for fun or even go to the movie theater which I love to do was the moment I realized something needed to change!
You are now equipped to be your own advocate- know your symptoms and your choices!
Fortunately, we now have more tools in our armament to make life better including a long list of medications one of which is the recently approved Gocovri (amantadine ER) for dyskenisias – however, because there are different types this may not be the answer for everyone. As an alternative, we now have 3 types of DBS surgeries or palladotomies, for those who live in countries where DBS is yet not available, which you can discuss for your physicians.
” I regret less the road not taken than my all fired hurry along the road I took.” Robert Brault
Many years ago when I first began this journey with Parkinson’s disease as a young doctor there were very few choices in the treatment of PD. since then there has been an explosion of new treatments and many are on the way. The news are a god sent like a much needed rain to a dry and hardened soil. However, they can also cause a lot of turmoil and stress for those of us living with the illness.
Knowing which medicine or treatment to choose and how to use it to get the most efficacy is still a challenge for both patients and physicians. but, in many ways this is a good problem to have rather than no choice at all. As new treatments become approved the possible combinations for treating a single individual increases exponentially. Which means that for us patients we not only have more options but also need to be more patient and willing to try many different medications and combinations to find the right one- this may take some doing and above all TIME! Plus, you and the treating physician need to well versed in these new treatments. In order to accomplish this feat now more than ever the presence of a specialist i.e. a movement disorder neurologist at the helm is crucial.
Both as a patient and physician I have learned that 1) you must have intricate knowledge of a formulation in order to use most effectively plus 2) have intricate knowledge of the person we are dealing with in order to be able to match the two in a positive fusion. This means that sometimes you have to be willing to use a bit of unconventional treatments requiring multiple doses, frequencies and medications to achieve the best outcome possible. I am a primary example of this fine tweaking resulting in my Parkinson’s being extremely well controlled for someone who has lived with it for more than 10 years. The first step to achieving this is being well informed as a patient in order to make the best decision possible given what we know at present. I believe that being a neurologist whose field of interest is PD puts me at a greater advantage but this is something that can be achieved by everyone.
You must start with knowing yourself and your body function. For instance, many people have asked why I have not head DBS especially since I am young and there is good data showing that early DBS is extremely beneficial decreasing disability. The earliest data indicates patients being stimulated after only 4 years of disease. I say to you as I have said to those people- although data is great 1) I am doing great with medication- no need to risk brain surgery although minimal risk is still brain surgery. 2) I am not a good candidate for DBS- I am a terrible surgical patient for many factors and more importantly as far as my PD I believe that my quality of life would be worst certainly if I were to do bilateral. I already have swallowing issues and balance issues both of which are known to worsen with bilateral DBS. plus, not to mention I am already overweight due to meds; studies have shown that women have a higher tendency to put on weight on average 20-30 lbs. I certainly do not need this. Although, I firmly believe DBS is a great therapy and is the standard of care but reality is that not everyone will do well. In order to maximize the benefits of this surgery patient selection is of the utmost importance. Always talk to your physician about the pros and cons and expectations prior to any surgical procedure.
The other thing is that it may take a year or two to find the right combination of medications and as disease progresses there may need to be another trial and error phase not as long but equally challenging for all involved and must be patient. This is not a race to the finish line rather a slow and steady way of life. Do not discard a medication from your tool box because it ‘did not work,‘ it ‘gave you side effects,’ or because it ‘stopped working’. First, we have to ask what is the medicine treating? is it the tremors? the pain? the stiffness? once we know we are better able to access its function. Most PD medications do not target all the symptoms motor or non-motor for sure. some are better than other at working a specific function. this is why most often in order to have the best outcome one must take a cocktail of pills as I do. also important to note that the Mao inhibitors like Azilect in particular usually do not cause dramatic effects unless looking at two specific things- 1) gait – it improves balance and 2) pain and 3) visual problems so if you have neither of these issues most likely not going to notice a difference. However, I and many of my colleagues believe there is a neuroprotective component to this drug making it more than worthwhile to take. further, it has been my experience that although it is a once a day drug it does not typically last more than 12 -14 hours hence I recommend taking it twice a day. we just got approval of a new MAO B inhibitor Xadago which also has glutamate reduction approved for off episodes. We will see how this medication plays out. but the fact that it targets two receptors is a better potential treatment.
All of the dopamine agonists Mirapex, Neupro, Requip can cause sedation, impulse control, and increase sexual urges as well as hallucinations in those that are prone to dementia or have dementia. Mirapex has the most sedation, impulse control and sexual impetus followed by requip. So it is important that if you are already prone to daytime sleepiness or have gambling problems or are ultra sexual that you talk to your physician about not using Mirapex or Requip unless as last resort. Also note that these medicines usually loose efficacy around 10 years so may need to take a small hiatus ( 6months to a year) and return. I prefer Neupro because does not cause sedation, OCD, nor worsen dementia. but, it can cause water retention especially in women. I myself alternate the neupro patch 2mg with 4mg. work great so sometimes have to find the right dose and is not a conventional same daily dose. these are fairly good for tremors, stiffness, and slowness as well as for restless legs, rem behavior, bladder control, mood (some),
The dopamine where levodopa –Sinemet is the gold standard of treatment. We also have Stalevo, Rytary and Duopa. these compounds approximate the natural substance so much better hence the effect is the best particularly when it co0mes to mood, memory and cognition in general. when people have trouble concentration , focusing, multi-tasking, learning, enjoying thins this signifies a deficiency in this chemical. what I have discovered is that levodopa in what ever form is what is required to maintain these capabilities without it we may control, tremors, stiffness, walking but we are going to feel awful, moody, cranky, fatigued, uninterested, have poor concentration and memory in fact you wont feel like you any more even if you look good and are able to do everything. of course this is the one associated with dyskinesia so people are afraid to start and even doctors don’t want to start early sometimes because of this fear which in the old days was much more certain to occur and start sooner. However, with all the medications at our disposal there is no reason to delay taking medication and even small doses of levodopa so you won’t feel like a zombie or sub-human. I started on regular Sinemet but caused lots of nausea then went to extended release which really did not last much longer but was last nauseating then because I needed higher doses and trying to keep risk of dyskinesia I switched and I absolutely love this drug because so many doses and don’t have so many peaks and through and again less GI and orthostatic symptoms. as my symptoms have advanced I could not extend Stalevo to higher doses without causing daily migraines – not worth the pain. thus when Rytary came out, I was first in line to try. I too love this drug and has a benefit unlike the others that I believe it mimics more the natural brain chemical release because for the first time since I got ill I felt like “me!” even my doctor who has known me since residency has stated that I was back!
A couple of side notes on Rytary which are my own personal observation and opinion but merit looking further into. First, I find that this drug is much more constipating than any of the others so have to be ware of maintaining regular bowel schedule. I had hear and read that some people experienced chest pain and arm pain with this drug. I was on it for over a year before I began to have lots of shortness of breath and chest pain with medication- turns out caused by severe hypertension. once we added more blood pressure medication I am able to tolerate Rytary once more. so, if there is family history of heart attacks, stroke especially since women seem to have higher risk of developing strokes after PD may want to talk to your doctor about concerns, monitor blood pressure regularly take prophylaxis for stroke and heart (these risks increase as we age as well). also follow with a cardiologist regularly I do. This medicine may be best suited for people with low blood pressure. nevertheless, if you have high blood pressure as I do does not mean you cant take it means more careful and precise control of PD meds and high blood pressure. I find that taking the medicine staggered works best but I would not recommend doing this on your own without talking to your physician. what I mean is that the recommended dose is 2 tablets twice, three times a day etc. but I discovered among my friends who were placed on this medication at the same time I was at similar doses having disease approximately same duration of time and they are my age, that after a year’s time all developed severe dyskinesia; only difference between them and me is gender ( 4 men; 1 woman). All these people have undergone or are waiting to have DBS. about 6 months ago I began experiencing mild dystonia/dyskinesia which I first attributed to having had decrease of meds due to blood pressure issues. however, once I restarted my previous regimen I quickly noticed that when Rytary wore off I was having the problem. so I began taking them in tandem 1 tablet then 4-6 hours later the second tablet and voila no more dyskinesia and I feel wonderful. prior to this I had consider adding Comtan with it to extend duration.
As you can see even for an expert like myself, there is a lot of trial and error and fine tuning. even addition of medication like amantadine which at one point I could not take because it triggered psychosis. however, our bodies are always changing, our disease is evolving and the illnesses we have at one point may improve or worsen interfering with PD treatments. For instance if you have H. pylori this will cause much more nausea and vomiting than usual plus will render Sinemet less effective – thus if something changes suddenly or dramatically from status quo need to speak to your physician.
In the end knowing your own body, being informed about medications, and having a good rapport with your physician will allow you to make the best informed decision about what treatments are best. Always knowing the end game helps plus another thing that Is a crucial or even more so than the treatment is having continuity of care with the same physician. Only then can they truly give you the best choices available based on your own uniqueness. Inadvertently, sometimes we sabotage ourselves by hoping from doctor to doctor which only creates confusion, unnecessary repeat testing and much disruption and frustration to your own life; because first you don’t allow enough time to build an appropriate patient physician rapport which would guide the specialist to making the best optimal decisions on your behalf . Second the constant change means a change in medications most of the time because each one of us is like an artist who sees the big picture and end result and we work in our own way to achieve that- but each physician like the artist has a different picture in mind- only leaving you the patient completely dumfounded and unwilling to try new things or see the one doctor who potentially could bring the masterpiece together with all the broken pieces discarded by everyone else.
Since both my mother and grandmother have been found to have brain meningiomas, I have begun to take a very serious look at this matter. My search has lead me to find a few cases of meningioma – benign brain tumor arising from the lining covering of the brain (the meninges) presenting as reversible Parkinsonism. However, my concern is the relationship between PD and an increase risk for occurrence of meningioma’s of the brain after PD diagnosis.
My grandmother as you might remember had Parkinson’s disease which began in her late 60’s. She had good response to PD meds for many years but then began having uncontrolled bladder issues first believed to be due to PD but the symptoms were out of proportion to disease state. It was then thought that the problem was due to Diabetes. Then came the increase falling and worsening vision. This continued until one day she suddenly developed hemiparesis and seizures which led to the discovery of an extremely aggressive meningioma of which she died of.
My search has lead me to a possible link of an increased incidence of ‘benign tumors’ (non- aggressive and normally do not spread to surrounding tissues; although they can be serious and life threating) with PD, this according to a large Taiwanese PD study. The incidence appeared to be an increase of 2.2. Furthermore, the incidence of benign tumors was higher in women with PD compared to women without PD especially in those over 50 years of age. However, the problem is that they never mentioned which ‘benign’ brain tumors?
The most common ‘benign’ brain tumor in adults are pituitary adenomas after gliomas, meningiomas, and schwannomas. So it would stand to reason that some of these benign tumors are indeed meningiomas.
What does this all mean for us?
Meningioma’s could be a possible treatable cause of Parkinson’s disease (parkinsonism)- these can present anywhere in the brain. Hence, the importance of obtaining an MRI of the Brain when first diagnosed. These occur more commonly in women ages 30-70; so YOPD women should always look, as any other PD patient, look at other treatable causes.
Head injury (controversial)
Increase in progesterone (post- menopause
Neurofibromatosis type 2 (genetic disorder)
Exposure to radiation
2. Meningioma’s are a possible increased benign brain tumor in PD patients especially in women. Therefore, it is important to keep in the back of your mind and discuss with your physician if new symptoms arise especially if not responding to PD medications or occurring out suddenly or out of frame with expected PD course. This is a time to have a repeated MRI/CT of the Brain. MRI is recommended because they usually begin to have calcifications or changes in the bone and arise in areas where there are a lot of bony structures making it easier to overlook. Because these grow very slowly over time usually they present with gradually developing symptoms.
Symptoms: the hard part is that some of these symptoms are part of PD – thus we have to remain hypervigilant especially when not obtaining the desired affect from medication. (Not responding well)
Blurred vision/loss of vision/
Weakness in arms or legs
(increased bladder/bowel function due to swelling when there is swelling of the brain)
3. Because benign brain tumors incidence has been reported to be increased in PD patients – until we have more defining studies even when all seems well maybe worthwhile to discuss with your physicians about an updated brain imaging to make sure some of the slow growing treatable tumors don’t sneak up on us. Furthermore, those of us who like me are LRRK2 carriers might be also at higher risk of another benign brain tumor a glioma.
Now, for a woman that was once proud of her brain it has been a big blow to watch my brain shrink since this disease took over me along with the realization that I may be at higher risk of having a brain tumor due to my genetics and family history. But, I will persevere and continue to fight and educate others in hope of a better tomorrow for all of us.
“The only time to eat diet food is while you’re waiting for the steak to cook.” Julia Child
As we start a New Year, thoughts of health and staying healthy seems to be one of the foremost concerns in most of our minds, at least they are for me. Most of us who have lived with PD for a while realize what a burden it can be especially in light of the fact that for the majority of us Parkinson’s is not the only disease we have or will ever have. Unfortunately, not only are we still at risk for developing other major illnesses as we age but PD itself being a systemic illness can in turn predisposes us to other diseases like various types of cancers, dementia, strokes, possible diabetes along with other gi problems. Thus, we must always be vigilant for any new symptoms. We must be savvy in recognizing these as well as knowing what to do when they do arise.
One of the best tips I can give you as a neurologist is to make sure that you have frequent follow up appointments with your MDS or neurologists especially if you have had PD for more than 5 years because sometimes we as patients are not very good at picking out subtle changes or worse when we do recognize there are changes we sometimes get afraid of the implications so we don’t bring it up and try to deal with it. You should see your physician upward of 4 times a year in my experience if you are more than 5 years certainly more than 10 years. Remember-Our body’s change as we change and so does our response to the medications.
Now that I am nearly 10 years into the disease I have noticed increased chocking and swallowing problems. When you choke on your own saliva –that is an attention grabber! The other day I choked while taking my potassium which resembles a “horse” pill. This nasty pill went down the wrong pipe, I was beginning to panic since it was stuck smack down the middle of my throat then I remember that the potassium pill dissolved quickly with water so even though I was chocking and gasping for air I was trying to drink water to dissolve pill. Of course it was making me panic more as I was really struggling to breathe…I thought is this how I die? Flash of a patient that asphyxiated eating a peanut butter sandwich passed quickly by…
I was not going to go down this way I was about to attempt a Heimlich maneuver when I began spewing and foaming the potassium out of my mouth in a violent cough. All because my PD meds had not kicked in before I took such large pill. This was too close for comfort, this meant time to adjust medications. In my case it was an easy solution but is not always straight forward.
If you are experiencing any amount of swallowing dysfunction even if mild does not have to be as violent or severe as mine to bring attention to the problem.
I am choking you and your doctor will both ponder if your PD has worsened?
Is it Parkinson’s or something else entirely?
First, you need to keep a record of the events:
When does it happen? Morning? Night? After medication intake or medication wears off?
Does it happen every day?
Does it happen with solids? Liquids? Or both?
Does it happen only with pill intake?
Do you cough? / wheeze?
Are there other symptoms with it like dizziness, unsteady gait?
Then you have to address:
Are meds not working? Are they wearing off? Need larger doses?
Do I have any other medical reasons for this? (strokes, gi problems, throat tumors)
Also if you had DBS implant you may have worsening of swallowing especially if already had some symptoms prior to surgery. sometimes adjustment of DBS can improve symptoms.
Fortunately, mine got corrected with adjustment of medications and addition of new med called RYTARY – intermediate release levodopa.
It is important to remember that even if there is aspiration things are not as black and white. I am glad that many other doctors and therapists are starting to take note of this fact. Food and being able to eat and enjoy it is a big part of our culture as well as our quality of life. Being able to taste and sit at the table makes us feel more like a normal person. So sometimes, even though a patient can’t swallow or is aspirating and requires a feeding tube –the family can work with the doctors and speech therapist top provide quality and comfort to their loved ones- may start with ice chips or food to taste like pudding, or a combination of being able to eat normal meals at certain times and use the peg tube at other times for pills or calorie sustenance.
Regardless of cause all patients with swallowing trouble, everyone should have an evaluation by a speech therapistwhich should include a modified barium swallow to make sure there is no aspiration in particular silent.
Your MDS/neurologist may refer you to get an ENT evaluation and/ or Gi evaluation to make sure there are no other treatable causes. They may also order MRI’s of brain or neck.
Usually swallowing difficulties in Parkinson’s may begin to occur after several years of illness usually >7 years, if having problems swallowing at the beginning or more pronounced and rapidly deteriorating is a big indicator that we are dealing with a more aggressive Parkinson’s plus type such as PSP, MSA, LBD etc.
These are all the questions that you and your doctor will need to address. You need to seek immediate attention if having difficulty swallowing in order to protect airway and prevent aspiration pneumonia which can lead to hospitalization and early demise.
Next time you feel stressed or that the passion and flair has gone out of your life because of PD, “Pull up a chair. Take a taste. Come join us. Life is so endlessly delicious.” (especially if you taste anything chocolaty!)
― Ruth Reichl
“Shaking is great. Shaking is one of the oldest practices known to man…connecting the earth and restoring energy through Out…” Jerome Flynn
Is my tremor a sign of PD? This is always the million dollar question on everyone’s mind when they go see a Movement disorder specialist for the first time. Although, this is the one tremor everyone ruminates on it is not the most common of the movement disorders nor the only tremor.
There are many other tremors associated with other neurological and medical conditions w hich can present challenges for daily living for those experiencing tremors independent of etiology.
Therefore, I want to spend a little bit of time talking about some of these and in particular the more common type of tremor also known as “essential tremor” since this seems to be one of the main reasons for which people are referred to a neurologist. Many of these usually have been misdiagnosed as having PD . It is estimated that about 20% of people with ET are labeled as having Parkinson’s according to a study by Henderson et al.(1995)
Essential tremor (ET) unlike Parkinson’s tremor is a postural, action tremor that progress slowly over many years. It is significantly more common than rest tremor of PD. In 60% of those with ET have an autosomal dominant trait. This disease can start early in life but it is much more common in older individuals due to increase in frequency due to age. There is an anticipatory mechanism with every generation making the tremors present sooner or at younger age than parents or grandparents. People with essential tremor can become severely disabled especially socially although diseases progresses very slow. Individual’s with ET usually are hard of hearing and have bilateral tremors and can have great difficulty with activities of daily living. They are also at higher risk for developing Parkinson’s disease later in life. Sometimes they also have accompanying head tremors (mostly nodding -yes), voice tremors and at times orthostatic tremors –which I will discuss later.
The test you can do at home is to draw a spiral first with one hand then the other and watch for cauliflower like pattern. also draw a straight line between two other lines in a narrow space and if it looks like the line is an inching worm or squiggly line then you have action tremors. See picture: Also, even though handwriting is affected it is completely different than that of PD. It is shaky and messy. Furthermore, this type of tremor responds well to alcohol intake sometimes causing patients with severe tremors to become alcoholics. Subsequently, they too are misdiagnosed because physician’s erroneously assume they have tremors due to their drinking and not the other way around. Besides alcohol which is not really recommended is treatment with Mysoline, Neurontin, Klonopin, Topamax, Beta-blockers -which I do not recommend in young people especially men due to tendency to cause severe depression besides lowering blood pressure. Botox works well if focal, but DBS to thalamus is best treatment plan if symptoms are severe.
Parkinson’s (rest) tremor, the second most frequent type of tremor has a much slower amplitude as if sending a ‘Morse’ code. It is often described as -‘pillrolling’ tremor present primarily at rest and starts unilaterally unlike ET. PWP’s often feel like they have to hide their tremors by putting their hands in their pockets when walking or sitting on their hands if at rest as to not draw attention to themselves. Person’s with rest tremor can do normal action specific maneuvers such as shaving or putting on mascara, without much if any interference. Occasionally, PD individuals can experience action tremors but these are different from above tremor in that the frequency is same as rest tremor. When asked to do above diagrams, PWP’s exhibit normal patterns- smooth and even! The handwriting is also affected as you know with PD but instead of messy is small and tight which usually gets smaller and tappers down as we write.Best treated with Anti-cholinergics – Artane & Amantadine and to a lesser extent dopamine replacement- Best results is DBS.
Another commonly seen tremor is Dystonic tremor. This tremor is said to be a forme fruste of ET. The best example of this is in patients who have cervical dystonia (i.e. spasmodic torticollis) who have a tendency to turn their head involuntarily in a particular direction say to the left and when they try to maintain their head and neck in a neutral position their head will shake involuntarily. One simple way to differentiate this tremor from essential tremors is a clear response and decline in tremor when a person uses antagonistic gestures. This means that the tremor improves through touch of the head or by lifting an arm etc. ET will not respond to antagonistic gestures!!Best treated with Klonopin and Botox.
Orthostatic tremors- Unique tremor syndrome more common in elderly and middle aged. This usually presents as a feeling of unsteadiness or dizziness while standing still. It typically does not interfere with gait unless is severe. the shaking or tremors are concentrated to the trunk and legs occasionally the arms. Best treated with Klonopin.
Cerebellar tremors- Unilateral Intention tremors but unlike essential tremors these patients have trouble with reaching target; not only do they shake as they get closer to target but when asked to do ‘finger to nose’ they overestimate or overshoot distance. Best treatment for this is Isoniazid, Tegretol, Mysoline.
Physiologic tremors. We all have very fine tremors of fingers especially pinky and can be visualized to naked eye. frequency is not as fast as those with ET- much more subtle and fine. yet they can increase under certain conditions such as stress, increased caffeine intake, abnormal metabolic disorders like diabetes and thyroid disease to name a few. Treatment finding root of problem. too much caffeine consumption is usually a big factor but these will present with other non- neurological medical problems.
Although, there are many other less frequent tremors these are the most common type. All tremors should be investigated and evaluated by a neurologist or MDS; so that the correct diagnosis and treatment can be initiated on a timely basis before there is interruption of function.